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Review Polyarteritis Nodosa Revisited Review Polyarteritis nodosa revisited: a review of historical approaches, subphenotypes and a research agenda O. Karadag1,2, D.J. Jayne1 1Department of Medicine, University of ABSTRACT However, the majority of PAN cases Cambridge, United Kingdom; Polyarteritis nodosa (PAN) is a rare reflect a variety of phenotypes of un- 2 Hacettepe University Vasculitis Centre, form of primary systemic vasculitis known cause and lack a system of phe- Ankara, Turkey. with heterogeneous presentations, treat- notypic subclassification. PAN can oc- Omer Karadag, MD ments and disease course. Historical cur at any age with wide variability in David J. Jayne, MD approaches to classification and diag- organ involvement, although survival Please address correspondence to: nostic terminology are reviewed. Since and recommended treatment strategies Prof. Omer Karadag, differentiation of PAN from microscopic Hacettepe Universitesi Tip Fakultesi, broadly reflect severity. We propose that Romatoloji Bilim Dali, polyangiitis (MPA) and other ANCA vas- subphenotypes within PAN having dif- 06100 Sihhiye-Altındag, culitides by the Chapel Hill conference ferent clinical courses may be different Ankara, Turkey. statements, and with hepatitis associated diseases rather than a spectrum of the E-mail: [email protected]; PAN defined as a secondary vasculitis, same disease. There is a need to better [email protected] the phenotyping and subclassification of understand these subphenotypes to un- Received on December 27, 2017; PAN has received little attention. Mono- derpin further clinical and translational accepted on January 17, 2018. genic disorders similar to PAN have been research and improve patient outcomes. Clin Exp Rheumatol 2018; 36 (Suppl. 111): described (familial Mediterranean fever, This paper aims to assess the heteroge- S135-S142. adenosine deaminase-2 deficiency), and neity of the clinical spectrum of PAN © Copyright CLINICAL AND cutaneous PAN and single organ vascu- and highlight differences between sub- EXPERIMENTAL RHEUMATOLOGY 2018. litis, discussed. The overlapping pheno- phenotypes. After reviewing the his- types between PAN and other primary torical evolution of the classification of Key words: polyarteritis nodosa vasculitic syndromes and subphenotypes PAN, approaches to diagnostic termi- (PAN), vasculitis, hepatitis B virus within PAN are explored. This work will nology, and the similarities and differ- related PAN, familial Mediterranean underpin development of newer treat- ences of subphenotypes will be consid- fever (FMF), deficiency of adenosine ment regimens and future genetic and ered. This will form the basis for a rec- deaminase 2 (DADA2) related aetiologic studies. ommended research agenda, such as, further histopathological evaluation of Introduction specimens and autopsy materials and a The term peri/polyarteritis nodosa has genome wide association study in PAN. been used for more than 150 years as a diagnosis for patients with primary Historical background systemic vasculitis defined by the pres- Rudolf Virchow proposed that vascu- ence of a necrotising arteritis of mus- litis might occur in blood vessels and cular, ‘medium sized’ arteries with or originate in the adventitia in a treatise without aneurysms. However, advances on histopathology in 1847 (3, 4). In in clinical and laboratory phenotyping 1852, Rokitansky described mesen- have led to specific syndromes, such teric aneurysms in the branch points of as microscopic polyangiitis (MPA) be- arteries in an autopsy case of polyarte- ing separated from an inclusive PAN ritis (5). Then Kussmaul and Maier in- categorisation. In parallel, there have troduced periarteritis to the literature been improvements in understanding of with the autopsy report from a 27-year certain causes of PAN, with hepatitis B- old tailor’s journeyman (6). related PAN defined as a ‘Vasculitis As- “A peculiar mostly nodular thickening sociated With Probable Etiology’ (1), (periarteritis nodosa) of countless ar- and the association of the monogenic teries and below the caliber of the liver disorder- Deficiency of Adenosine artery and the major branches of the Deaminase 2 (DADA2) (2) with a PAN coronary arteries of the heart, princi- Competing interests: none declared. phenotype (Fig. 1). pally in the bowel, stomach, kidneys, Clinical and Experimental Rheumatology 2018 S-135 Definition of polyarteritis nodosa-subphenotypes / O. Karadag & D. Jayne and biopsy of a small or medium-sized artery containing polymorphonuclear neutrophils. This study described four PAN subsets according to clinical features at presen- tation: • Positive arteriogram with abnormal AST or ALT; • Positive arteriogram, male sex; nor- mal AST and ALT; • Positive artery biopsy and neuropa- thy; negative arteriogram; • Neuropathy and weight loss >6.5 kg, negative arteriography and ar- tery biopsy. Because this classification system did not include a category for MPA, MPA cases would have been included in the PAN or hypersensitivity vasculitis cohorts. Proposal of an international Fig. 1. Schema representing etiologic/genetic factors and subgroups of poliarteritis nodosa (PAN). consensus conference on nomenclature Less studied/related subgroups are shown in purple. of systemic vasculitides, Chapel Hill (CHCC) (13) spleen, heart and voluntary muscles, autoantibodies against extra-nuclear The name “polyarteritis nodosa- and, to a lesser extent, also in the liver, components of polymorphonuclear (PAN),” or alternatively, the name subcutaneous cell tissue, and in the granulocytes were detected in serum “classic PAN,” is restricted to disease in bronchial and phrenic arteries.” samples from patients with Wegener’s which there is arteritis in medium-sized Because inflammation was not confined granulomatosis (now granulomatosis and small arteries without involvement to the adventitia, and primary changes with polyangiitis) and called anti-neu- of smaller or microscopic vessels. were in the media, Dickson suggested trophil cytoplasm antibodies (ANCAs) The proposed distinguishing feature the name, polyarteritis nodosa (PAN) (11). The association of ANCA with a for PAN versus MPA, is the absence (7). In the 1930s, approximately 150 pauci-immune vasculitis and evidence versus the presence of vasculitis in ar- cases of PAN were reported, 20 from supporting the pathogenetic role of terioles, venules, or capillaries. By this the US and mostly from autopsies (8). ANCA led to the definition of ANCA definition, involvement of “microscop- They believed that any organ or combi- associated vasculitis as an aetiologic ic” vessels must be present in micro- nation of organs may be affected at any and phenotypically separate vasculitis scopic polyangiitis, although medium- time in the course of the disease, and subgroup. sized or small arteries can also be in- the resulting clinical manifestations volved. In contrast, PAN must have no may be bizarre in the extreme. Meetings and statements for involvement of “microscopic” vessels, As the nosology and nomenclature of classification/diagnostic criteria and including no glomerulonephritis. Ad- the systemic vasculitides became better nomenclature of vasculitides ditionally, mucocutaneous lymph node defined, PAN became less frequently The American College of syndrome was mentioned as the defin- diagnosed. Many different forms of Rheumatology (ACR) 1990 criteria ing feature that sets Kawasaki disease vasculitis were described. By the early for the classification of polyarteritis apart from PAN. 1950s, hypersensitivity vasculitis was nodosa (12) distinguished from polyarteritis nodosa According to this classification sys- The European League Against (9). Afterwards, necrotising vasculitis tem, patients with systemic vasculitis Rheumatism (EULAR)/Paediatric confined to the skin without systemic were classified as PAN if they had least Rheumatology European Society involvement – Cutaneous PAN – was three of 10 criteria: weight loss above (PReS) endorsed consensus criteria described and distinguished from PAN 4kg, livedo reticularis, testicular pain/ for the classification of childhood in 1974. In the early 1970s, studies by tenderness, myalgia, weakness or leg vasculitides (14) several authors suggested chronic hep- tenderness, mononeuropathy/polyneu- New updated classification scheme atitis B (HBV) infection could cause ropathy, diastolic blood pressure above split the old term PAN into classical PAN (10). 99 mmHg, elevated urea and creati- PAN, cutaneous PAN, MPA and HBV- In the 1980s immunoglobulin G (IgG) nine, HBV, arteriographic abnormality, related PAN. S-136 Clinical and Experimental Rheumatology 2018 Definition of polyarteritis nodosa-subphenotypes / O. Karadag & D. Jayne To classify a child as having PAN re- quired a biopsy showing a small and mid-size artery necrotising vasculitis or angiographic abnormalities (aneu- rysms or occlusions) in addition to at least two of the systemic features (skin involvement; systemic hypertension; mononeuropathy or polyneuropathy; abnormal urine analysis and/or im- paired renal function, testicular pain or tenderness, signs or symptoms sug- gesting vasculitis of any other major organ system-gastrointestinal, cardiac, Fig. 2. MR Angiography of a patient with PAN (Courtesy of Hazirolan T, Professor in Hacettepe pulmonary, or central nervous system) University Department of Radiology, Ankara-Turkey) (CT Coronal MIP image (A), volume rendered are required. Magnetic resonance an- image (B). There is a microaneurysm at the lower parts of right kidney. And there are multiple
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